Case files 3 Flashcards
2 big signs of DMD
Waddling gait and progressive proximal muscle weakness (pushes off floor to stand up –> Gower sign)
2 initial DMD tests
Serum CK (elevated from muscle destruction)
DNA analysis of blood
Serum CK can also be elevated in females who are carriers
When does DMD begin to act
3-5 years, normal before that
Wheelchair dependent by 10-13 years
All DMD patients have some degree of? What is often the cause of death?
Cardiomyopathy
Respiratory failure is often the cause of death
Atopic dermatitis features
Pruritic, recurrent, and flexural in older children and symmetrical in adults
3 waves of AD
Infant: 12 - near onset of puberty
Seborrheic dermatitis limited to? Tx?
Areas of the skin with high concentrations of sebaceous glands (cradle cap)
Tx is ketoconazole
Brown and not weepy like AD
Why are fluorinated steroids avoided on face
May depigment and thin the skin
Kid with hives likely etiology
Recent antibiotic use
Cutaneous herpes simplex in infants likely found in
Areas or recently healed or active atopic dermatitis
Thrombocytopenia, Immunodef, and eczema
Wiskott-Aldrich
look for infections and bleeding in first six months of life
Baseline therapy for atopic dermatitis
Avoidance of drying soaps and replenishment of skin hydration with emollients
steroids may be added
Paraneoplastic syndrome related to
tumor production of catecholamines and VIP
What is a neuroblastoma made of? Where does it met?
Primitive neuroendocrine tissue
Mets to long bones, skull, lymph nodes, liver, and skin
Blueish skin in neuroblastoma in infant represents? Other major sign
Subcutaneous infiltration
**Raccoon eyes: proptosis and bluish periorbital discoloration
Wilms tumor features
Hematuria, hypertension, and localized abdominal mass that RARELY crosses the midline (unlike neuroblastoma - generally younger and sicker)
Neuroblastoma labs
Elevated VMA and HVA levels
Palpable abdominal mass in newborn likely
Hydrfonephrotic or multi cystic dysplastic kidney –> US for workup and dx
In baby intestinal obstruction (bilious vomiting), distention, blood per rectum and lethargy likely? Next step?
Malrotation with volvulus
Call surgery to remove necrotic bowel and reperfuse
Most common site for intussesception
Ileocolic portion of bowel
Ligament of treitz attaches to right side instead of normal left
Electrolyte levels in hypertrophic pyloric stenosis
hypochloremic metabolic alkalosis
What distinguishes NEC from malrotation
Pneumatosis intestinal (air in wall of the small bowel)
Current jelly stools in kid
Intussusception
Most common cause of severe urinary tract obstruction in boys? Workup?
Posterior urethral valves
Workup: Voiding cystourethrogram or USG and bladder cath, surgery after stabilization
Test for any young male(
Anatomic workup
PUV risks
25%-30% risk of ESRD
Injury when child pulled by arm and holds arm close to body with elbow in flexed position? what else will child do
Subluxation of the radial head (occurs when sudden traction on extended arm)
Child will also resist passive movement of the arm
How will child hold arm in subluxation of the radial head? PE?
Will hold the affected arm close to their body with the elbow flexed and forearm pronated
Absence of bony tenderness and swelling on exam